Vasculitis Flashcards by Jing Ji

What are the layers of arteries?

endothelium (tunica intima)

smooth muscle (tunica media)

fibrous connective tissue (tunica adventitia)

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What are the types of vascular disease?

arterial diseases

venous diseases

lymphatic diseases

vascular tumors

congenital anomalies

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What are the categories of arterial diseases?

arteriosclerosis

aneurysm

vasculitis

functional arterial disease

fibromuscular dysplasia

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What is vasculitis?

inflammation of and damage to blood vessels

vessel damage leads to ischemia (usually predominates)** **or aneurysm which compromises end-organ function

heterogenous - many etiologies, many manifestations

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What are causes of secondary vasculitis?

infection

drugs

malignancy

connective tissue disease (lupus or progressive systemic sclerosis)

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What are do the primary vasculitides have in common?

autoimmune etiology

inflammation of the vessel wall often leads to necrosis or aneurysmal formation

vessel wall inflammation -> occlusion or aneurysm -> end organ dysfunction

constitutional symptoms (fever, weight loss, malaise, anorexia, fever)

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What are the subclassifications of vasculitis?

large vessel

medium-sized vessel

small vessel

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What are the large vessel vasculitis?

Giant Cell Arteritis

Takayasu’s Arteritis

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What are the medium-sized vessel vasculitides?

Polyarteritis Nodosa

Kawasaki disease

Thromboangiitis Obliterans

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What are the small vessel vasculitides?

Immune Complex Mediated

ANCA-associated

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What is the classic presentation of giant cell arteritis (aka temporal arteritis)?

patients > 50 yrs, men more than women

extracranial arteries, temporal is common

classic presentation: fever, anemia, high ESR, headaches

other common manifestations:

jaw claudication
polymyalgia rheumatica (pain and stiffness in the hips and shoulders)
involvement of the ophthalmic artery can lead to blindness

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How is the diagnosis of giant cell arteritis confirmed?

temporal artery biopsy

granulomatous inflammation - can see giant cells in vessel walls, which gives the arteritis its name

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What is the treatment for giant cell arteritis?

high-dose steroids such as prednisone

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What is the classic presentation of Takayasu’s Arteritis?

women < 50

asian descent

constitutional symptoms first (fever and weight loss) before clinical manifestation of clinical symptoms

involves ascending aorta and immediate branches

“pulseless disease” (decrease in peripheral pulse)

end-organ blood supply can also be affected (neurologic symptoms, claudication, or pulmonary hypertension)

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What are the histological findings of Takayasu’s Arteritis?

histology is the same as temporal arteritis

giant cells are present

inflammation across all 3 layers of artery

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What is the treatment for Takayasu’s Arteritis?

steroids

cytotoxic agents

anaotmic correction of stenosis - stents or bipass if needed

What are the common clinical findings of Polyarteritis Nodosa?

often middle-aged patients

small-medium sized muscular arteries

skin manifestations such as cutaneous nodules

Mononeuritis Multiplex (people have peripheral neuropathies because of involvement of arteries associated with no central nerve identifiable)

Renal: hematuria

30% associated with Hep B

GI manifestations: abdominal pain

What are the phases of Polyarteritis Nodosa?

acute phase - transmural inflammation and fibrinoid necrosis

chronic phase - fibrous wall thickening

stages can coexist!

What is the treatment for Polyarteritis Nodosa?

corticosteroids and cyclophosphamide

What is the common presentation of Kawasaki Disease?

young children (less than 4)

T-cell delayed hypersensitivity

endothelial antibodies precipitating acute vasculitis

Classic Pentad: fever, conjunctivitis, strawberry tongue, cervical lymphadenitis, desquamative rash

can lead to MI or death

What are the risks of leaving Kawasaki Disease untreated?

risk of coronary artery ectasia and aneurysms

What is the treatment for Kawasaki Disease?

high dose aspirin

100 mg/kg/day

intravenous immunoglobulin

What are the general features of Thromboangiitis Obliterans (Buerger’s Disease)?

medium-small arteries

very cellular, inflammatory thrombus with relative vessel wall sparing

young heavily smoking males

can involve nerve and vein

very segmental - leads to symptoms of claudication

tibial and radial arteries most involved

can lead to rest limb pain, ulcerations, and gangrene

What are the classic symptoms of Thromboangiitis Obliterans?

rest pain

instep claudication

Raynaud’s Phenomenon

Limb ischemia

superficial thrombophlebitis

What is the treatment for thromboangiits obliterans?

stop smoking likely that further artery involvement will cease patients may still be left with claudication

What is Raynaud's Phenomenon?

**triphasic change on cold exposure**: 1) hyperemia (fingers turn red) 2) vasoconstriction (fingers blanch) 3) blue coloration from long-term tissue hypoxia **Primary** - _Raynaud's disease_ if no cause **Secondary** - _Raynaud's Phenomenon_ if secondary cause

What is the treatment for Raynaud's Phenomenon?

avoid cold exposure calcium channel blockers

What are the ANCA-associated small vessel vasculitides?

Granulomatosis with Polyangiitis (aka Wegener's) Microscopic Polyangiitis Churg-Strauss Syndrome

What is Henoch-Schonlein Purpura?

small vessels children less than 10 **IgA deposition in capillaries and venules** necrotising lesions *Palpable purpura - buttocks, lower extremities, and over extensor surfaces of arms and legs* **Other symptoms**: - polyarthralgia without arthritis - abdominal pin - renal involvement - often antecedent URI-type illness

What is the treatment for Henoch-Schonlein Purpura?

usually self-resolving steroids can help prognosis is usually very good

What is ANCA?

Antineutrophil Cytoplasmic Antibodies can see in vasculitis and other autoimmune disorders perinuclear ANCA (anti-myeloperoxidase, MPO) and cytoplasmic ANCA (anti-proteinase 3, PR3)

What is granulomatosis with polyangiitis?

**two pathologic hallmarks**: 1) granuloma formation 2) necrotizing vasculitis of smalla rteries and veins middle-age patients and associated with c-ANCA

What is the classic triad of granulomatosis with polyangiitis?

**initial phase**: *upper respiratory tract* - mucosal ulcerations, frequent URIs, pain and purulent discharge, chronic necrotizing inflammation **chronic phase**: *Lower respiratory tract* (hemoptysis, dyspnea, pleuritic pain) and *renal involvement* (glomerulonephritis)

What is the treatment of granulomatosis with polyangiitis?

steroids or cyclophosphamide

What are the features of microscopic polyangiitis?

small vessels constitutional symptoms **major manifestation**: - renal involvement (nephritic syndrome, often RPGN clinically) - pulmonary involvement (pulmonary capillaritis and diffuse alveolar hemorrhage) p-ANCA pattern

What is the presentation of Churg-Strauss Syndrome?

1 in a million **classic triad**: 1) asthma, allergic rhinitis, nasal polyps 2) peripheral eosinophilia 3) vasculitis p-ANCA present in ~50% **Other manifestations**: - peripheral neuropathy: *mononeuritis multiplex* quite common - myocarditis from eosinophilic infiltrates - palpable purpura - renal involvement less common

What is the treatment for Churg-Strauss Syndrome?

corticosteroids, cyclophosphamide